Images in cardiovascular medicine: Giant right ventricular fibroma in an infant.

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HomeCirculationVol. 106, No. 3Giant Right Ventricular Fibroma in an Infant Free AccessReview ArticlePDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissions ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toFree AccessReview ArticlePDF/EPUBGiant Right Ventricular Fibroma in an Infant Massimo A. Padalino, MD, Cristina Basso, MD, PhD, Gaetano Thiene, MD and Giovanni Stellin, MD Massimo A. PadalinoMassimo A. Padalino From the Departments of Cardiovascular Surgery (M.A.P., G.S.) and Pathology (C.B., G.T.), University of Padova Medical School, Padova, Italy. Search for more papers by this author , Cristina BassoCristina Basso From the Departments of Cardiovascular Surgery (M.A.P., G.S.) and Pathology (C.B., G.T.), University of Padova Medical School, Padova, Italy. Search for more papers by this author , Gaetano ThieneGaetano Thiene From the Departments of Cardiovascular Surgery (M.A.P., G.S.) and Pathology (C.B., G.T.), University of Padova Medical School, Padova, Italy. Search for more papers by this author and Giovanni StellinGiovanni Stellin From the Departments of Cardiovascular Surgery (M.A.P., G.S.) and Pathology (C.B., G.T.), University of Padova Medical School, Padova, Italy. Search for more papers by this author Originally published16 Jul 2002https://doi.org/10.1161/01.CIR.0000018039.93248.F4Circulation. 2002;106:386Apreviously asymptomatic 5-month-old girl was admitted because of polypnea and dyspnea, with signs of moderate heart failure. Physical examination showed diffuse wheezing, a 3/6 systolic precordial murmur, and an enlarged liver 4 cm from the right costal border. Chest x-ray demonstrated an increased cardiothoracic index (0.52) with pulmonary congestion. ECG showed sinus rhythm. An episode of ventricular tachycardia (175 bpm) occurred soon after hospitalization. Two-dimensional echocardiography showed a huge, roundish mass (6×5 cm in size) at the right ventricular (RV) free wall site, which was causing RV outflow tract obstruction (49 mm Hg). MRI (Figure 1) confirmed a solid mass, 6×4×5 cm in size, involving the RV anterior myocardial wall and causing severe RV cavity reduction. Main pulmonary artery and aorta were cranially and posteriorly dislocated. Download figureDownload PowerPointFigure 1. MRI (A, axial plane; B, sagittal plane) shows a huge, solid mass, 6×4×5 cm in size, involving the RV anterior myocardial wall, causing severe RV cavity reduction and RV outflow obstruction.At cardiopulmonary bypass, the mass was finally isolated and resected, through gentle dissection of myocardium from epicardium and endocardium, and the RV free wall was reconstructed with a 0.6-mm polytetrafluoroethylene patch. An intraoperative biopsy showed abundant proliferation of fibroblasts with collagen deposition, which are diagnostic for cardiac fibroma (Figure 2A). The excised, rounded mass, 81 g in weight and 6×5 cm in size (Figure 2B), appeared firm, white, and whorled. Download figureDownload PowerPointFigure 2. A, Surgical biopsy of the RV mass showing abundant proliferation of fibroblasts with collagen deposition (trichrome Azan ×4). B, Intraoperative view. After starting total cardiopulmonary bypass, with deep hypothermia and aortic cross-clamping, the RV free wall epicardium was cut down to gently enucleate the mass. When the mass was completely isolated, it finally was resected.At follow-up 5 years later, the child is alive and well, free of medications, and has sinus rhythm. Two-dimensional echocardiography shows normal RV contractility with mild dilatation and hypertrophy.The editor of Images in Cardiovascular Medicine is Hugh A. McAllister, Jr, MD, Chief, Department of Pathology, St Luke’s Episcopal Hospital and Texas Heart Institute, and Clinical Professor of Pathology, University of Texas Medical School and Baylor College of Medicine.Circulation encourages readers to submit cardiovascular images to the Circulation Editorial Office, St Luke’s Episcopal Hospital/Texas Heart Institute, 6720 Bertner Ave, MC1-267, Houston, TX 77030.FootnotesCorrespondence and reprint requests to G. Stellin, MD, Department of Cardiac Surgery, University of Padova Medical School, Via Giustiniani, 2, 35128-Padova, Italy. E-mail [email protected] Previous Back to top Next FiguresReferencesRelatedDetailsCited By Delmo Walter E, Javier M, Sander F, Hartmann B, Ekkernkamp A and Hetzer R (2016) Primary Cardiac Tumors in Infants and Children: Surgical Strategy and Long-Term Outcome, The Annals of Thoracic Surgery, 10.1016/j.athoracsur.2016.04.057, 102:6, (2062-2069), Online publication date: 1-Dec-2016. Basso C, Rizzo S, Valente M and Thiene G (2016) Cardiac masses and tumours, Heart, 10.1136/heartjnl-2014-306364, 102:15, (1230-1245), Online publication date: 1-Aug-2016. Thiene G, Valente M and Basso C (2013) Cardiac Tumors: From Autoptic Observations to Surgical Pathology in the Era of Advanced Cardiac Imaging Cardiac Tumor Pathology, 10.1007/978-1-62703-143-1_1, (1-22), . Padalino M, Basso C, Milanesi O, Thiene G and Stellin G (2013) Primary Cardiac Tumors in the Pediatric Age Cardiac Tumor Pathology, 10.1007/978-1-62703-143-1_5, (59-71), . July 16, 2002Vol 106, Issue 3 Advertisement Article InformationMetrics https://doi.org/10.1161/01.CIR.0000018039.93248.F4PMID: 12119258 Originally publishedJuly 16, 2002 PDF download Advertisement